Harjola Marable Syndrome: a Rare Case Report

International Journal of Scientific and Research Publications, Volume 8, Issue 9, September 2018 137 ISSN 2250-3153

Harjola Marable Syndrome: A Rare Case Report

Shirish R Bhagvat, Mehdi Kazerouni, Suhas Parikh, Amol Wagh, Jalbaji P More, Prachiti S Gokhe

Department of Surgery, Wockhardt hospitals and Sir J. J. Group of Hospitals, Mumbai, India

DOI: 10.29322/IJSRP.8.9.2018.p8119 http://dx.doi.org/10.29322/IJSRP.8.9.2018.p8119

Abstract- Background: Compression of celiac artery due to Median arcuate ligament syndrome is difficult to diagnose unusual placement of median arcuate ligament which is and is a diagnosis of exclusion. It is usually diagnosed after ligamentous continuation of diaphragm over aorta results into extensive workup including upper endoscopy, colonoscopy, and symptom complex called as median arcuate ligament syndrome evaluation for gallbladder disease and gastroesophageal reflux or Harjola Marable syndrome. All the patients who have unusally disease (GERD). low lying median arcuate ligament may or may not be symptomatic. Case report: We describe a case of 31 years old male II. CASE REPORT patient who presented with complaints of pain in epigastric 31 years old male patient presented with complaints of region and was diagnosed as celiac artery syndrome after pain in abdomen, mostly in epigastrium on and off since 1 year, extensive workup including OGD scopy, USG and CT aggrevated since 6 months. Pain used to aggrevate on food intake angiography. He underwent laparoscopic release of median and after activity. Pain was associated with nausea and giddiness. arcuate ligament which resulted in relief of symptoms. Physical examination was essentially normal.Upper GI scopy was within normal limits.USG abdomen showed narrowing of Index Terms- Median arcuate ligament syndrome, celiac artery celiac artery at the origin with increased in peak systolic syndrome , Harjola-Marable syndrome ,Celiac axis syndrome, Velocity. Diameter of celiac artery at origin 3.3 mm and distal Marable syndrome celiac artery 6.6 mm suggestive of post stenotic dilatation. PSV in erect position 134 cm/sec on inspiration and 204 I. INTRODUCTION cm/sec on expiration.On supine position, PSV increased to eliac artery compression was first observed by Benjamin 304cm/sec.Above findings were suggestive of ? celiac artery C Lipshutz in 1917. It is also been called as Harjola-Marable compression syndrome. Which was further confirmed with CT syndrome, Marable syndrome, celiac artery compression angiography. CT angiography showed severe narrowing of the syndrome, celiac axis syndrome, celiac trunk compression origin of celiac artery due to thickened median arcuate ligament syndrome or Dunbar syndrome. of diaphragm. There is post stenotic dilatation of celiac artery. The median arcuate ligament is a ligament formed by Findings are suggestive of median arcuate ligament syndrome. joining of the left and right diaphragmatic crura join near the Patient underwent laparoscopic release of median arcuate 12th thoracic vertebra. This fibrous arch forms the anterior aspect ligament. Post op was uneventful. of the aortic hiatus, through which the aorta, thoracic duct, 5 months after surgery patient underwent repeat abdominal and azygos vein pass. The median arcuate ligament usually ultrasound which showed marked improvement in celiac artery comes into contact with the aorta above the branch point of PSV.USG with Doppler showed calibre of celiac artery at origin the celiac artery. Occasionally the median arcuate ligament and in distal part to be 5-6 mm. PSV in erect position on passes in front of the celiac artery,below T12, compressing the inspiration and expiration 113 cm/sec and 124 cm/sec in supine celiac artery and nearby structures such as the celiac ganglia. In position. some of these individuals, this compression is pathologic and PSV and diastolic velocity of celiac artery were in leads to the median arcuate ligament syndrome. expected range even in supine position and on expiration. No The syndrome most commonly affects individuals between elevated velocity seen in supine position and on expiration which 20 and 40 years old, and is more common in women. was suggestive of adequate release of median arcuate ligament. Patients with MALS experience abdominal pain, particularly in the epigastrium, which may be associated with eating and which may result in anorexia and weight loss. Other III. DISCUSSION signs are persistent nausea, and exercise intolerance. Diarrhoea is Classically, MALS involves a triad of a common symptom. 1.Abdominal pain after eating, Occasionally, abdominal bruit is heard in the mid- 2.Weight loss, and epigastrium. 3.An abdominal bruit. Complications of MALS result from chronic compression of the celiac artery. They include gastroparesis and aneurysm of the superior and inferior pancreaticoduodenal arteries.

http://dx.doi.org/10.29322/IJSRP.8.9.2018.p8119 www.ijsrp.org International Journal of Scientific and Research Publications, Volume 8, Issue 9, September 2018 138 ISSN 2250-3153

Although the classic triad is found in only a minority of individuals. And as the symptoms are non specific it is difficult to diagnose. There are various theories stating cause of pain in MALS. 1.Pain in MALS is assumed to be arising from inadequate blood flow through celiac artery due to Stenosis. 2.Compression of celiac ganglion. 3.Steal phenomenon which suggests diversion of blood to celiac artery from superior mesenteric artery through collaterals resulting in inadequate flow to intestine.

Although compression of celiac artery is widely accepted. MALS is diagnosed using duplex ultrasonography.It measure blood flow through the celiac artery. Peak systolic velocities greater than 200 cm/s are suggestive of celiac artery stenosis . Further evaluation and confirmation can be obtained via angiography to investigate the anatomy of the celiac artery.

CT angiographic findings in MALS s/o 1.Proximal stenosis of celiac artery with post stenotic dilatation. 2.Indentation on superior aspect of celiac artery Figure 1CT image on sagital section showing narrowing at 3.Hook shaped contour of celiac artery. celiac artery origin and its hook shape contour.

These imaging features are exaggerated on expiration, even in normal asymptomatic individuals without the syndrome. REFERENCES The hook-shaped contour of the celiac artery is [1] El-Hayek KM, Titus J, Bui A, Mastracci T, Kroh M. Laparoscopic median characteristic of the anatomy in MALS and helps distinguish it arcuate ligament release: are we improving symptoms? J Am Coll Surg from other causes of celiac artery stenosis such 2013 Feb;216(2):272-9. doi: 10.1016/j.jamcollsurg.2012.10.004. [2] Lindner, H.H., Kemprud, E. A clinicoanatomic study of the arcuate as atherosclerosis. ligament of the diaphragm. Arch Surg. 1971;103:600–60 Decompression of the celiac artery is the general approach [3] https://en.wikipedia.org/wiki/Median_arcuate_ligament_syndrome) to treatment of MALS. The mainstay of treatment involves an open or laparoscopic surgical approach to divide, or separate, the median arcuate ligament to relieve the compression of the celiac artery. Other modalities include stenting with dilatation AUTHORS although chances of recurrence are high, percutaneous First Author – Shirish R Bhagvat, Department of Surgery, angioplasty and aortoceliac bypass graft. Wockhardt hospitals and Sir J. J. Group of Hospitals, Mumbai, India Second Author – Mehdi Kazerouni, Department of Surgery, IV. CONCLUSION Wockhardt hospitals and Sir J. J. Group of Hospitals, Median arcuate ligament syndrome is a diagnosis of Mumbai, India exclusion in a case of pain in abdomen, especially in case of post Third Author – Suhas Parikh, Department of Surgery, prandial fullness. It should be considered in patients complaining Wockhardt hospitals and Sir J. J. Group of Hospitals, of post prandial pain in abdomen in whom other like GB disease, Mumbai, India pancreatitis, acid peptic disease have been ruled out. Fourth Author – Amol Wagh, Department of Surgery, Wockhardt hospitals and Sir J. J. Group of Hospitals, Mumbai, India Fifth Author – Jalbaji P More, Department of Surgery, Wockhardt hospitals and Sir J. J. Group of Hospitals, Mumbai, India Sixth Author – Prachiti S Gokhe, Department of Surgery, Wockhardt hospitals and Sir J. J. Group of Hospitals, Mumbai, India

http://dx.doi.org/10.29322/IJSRP.8.9.2018.p8119 www.ijsrp.org International Journal of Scientific and Research Publications, Volume 7, Issue 8, August 2017 139 ISSN 2250-3153